The value of malignancy evaluation in patients with dermatomyositis

The value of malignancy evaluation in patients with dermatomyositis

Clinical r e v i e w I I II I I The value of malignancy evaluation in patients with dermatomyositis Jeffrey P. Callen, M.D. Louisville, KY Derma...

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Clinical r e v i e w I

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II

I

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The value of malignancy evaluation in patients with dermatomyositis Jeffrey P. Callen, M.D.

Louisville, KY Dermatomyositis (DM) has been linked to internal malignancy in adult patients. However, the value of an extensive malignancy evaluation in patients with DM is controversial. Fifty-seven patients who had DM with malignancies, in whom data were available regarding the discovery of malignancy, have been analyzed. Fifty-three of these were reported previously. There were sixty-seven malignancies in the fifty-seven patients. The malignancy preceded (26 cases), followed (23 cases), or occurred with the DM (18 cases). A "blind" (nondirected) malignancy search was not of value in any of the cases analyzed. Rather, the tumors were discovered in forty cases by history (preceding tumor, or abnormal symptoms), in fourteen cases by physical examination, or in twelve cases by abnormal laboratory findings (chest x-ray, urinalysis, stool guaiac, etc). One case was not discovered until autopsy (adenocareinoma of the broad ligament). Analysis of tumor sites further negates the value of a malignancy work-up, because most (>90%) tumors occur in areas not amenable to a "routine malignancy search." In several instances patients had an extensive search, without having complete physical examinations. Malignancy evaluations should be directed by abnormal history, physical findings, or routine laboratory testing. (J AM ACAO DERMATOL6:253-259, 1982.)

Dermatomyositis (DM) has been linked with internal malignancy in 15% to 34% of adult patients.' In 1975, Bohan and Peter 2 questioned the validity of this relationship. They criticized the previous data because of a lack of predefined diagnostic criteria for the confirmation of myositis. Consequently, Callen et al ~ used the criteria suggested b y Bohan and Peter and found malignancy in 25% of adult patients with dermatomyositis. Talbott 4 reported additional cases of DM and

Partially presented at the American Rheumatological Association Annual Meeting, June 5, 1981; abstract in April, 1981, Arthritis and Rheuma}ism. Reprint requests to: Dr. Jeffrey P. Callen, University of Louisville School of Medicine, 323 East Chestnut St., Louisville, KY 40202. 0 1 9 0 - 9 6 2 2 / 8 2 / 0 2 0 2 5 3 + 0 7 5 0 0 . 7 0 / 0 © 1982 A m Acad Dermatol

malignancy, and restressed the importance of this relationship. Callen et al and others 2-6 have questioned the validity of extensive malignancy evaluation in patients with DM. In our group, malignancy was not found in any case by the use of extensive radiologic or surgical procedures in otherwise asymptomatic DM patients. Moss and Hanelin 5 similarly confirmed the lack of value of radiologic screening tests. Despite these observations, controversy still exists regarding the value of screening DM patients for malignancy. Rowland et al, 6 Moss and Hanelin, 5 Callen et al, a Talbott, 4 and Bohan and Peter 2 questioned the use of screens. On the other hand, Bluefarb et al, 7 Barnes, t and Tuffanelli 8 suggested that a malignancy search 253

254

Journal of the American Academy of Dermatology

Callen

T a b l e I. D e r m a t o m y o s i t i s and m a l i g n a n c y Author

Age

II

Sex

1. 2. 3. 4, 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15.

Harris et aP Kellogg and Talley 1° Moss and Hanelin s Moss and Hanelin s Calvert and Neale 11 Rapoport and Omenn TM TalbotP TalbotP Talbott 4 Vesterager et alia Vesterager et a113 Vesterager et aP a Vesterager et a113 Lintum and Goedbloed 14 Ko et at t5

48 44 68 53 45 76 37 53 55 ? ? ? ? 67 63

F F F F F M F F F ? ? ? ? F F

16. 17. 18. 19. 20. 21. 22. 23. 24.

Allan et al 1~ Callen et alz Callen et al3 Callen et alz Schwarz et aP s Schwarz et al~2 Schwarz et alas Schwarz et alas Present study No. 1

41 38 45 58 ? ? '~ ? 52

F F F M F F F M F

25. Present study No. 2

67

F

should be extensive. This study was designed to re-evaluate the data in the literature on which m a n y authorities base their c o n c l u s i o n to advise extensive evaluation. METHODS

This series is based on a review of the English literature in which dermatomyositis was associated with malignancy and four additional cases not previously reported. In each case, a review of the data on which the diagnosis of DM was based was made, and if definite or probable DM (as previously defined by Callen et al a) could not be confirmed the case was not included. In addition, cases were excluded from the series in which data were not available regarding other symptoms or signs which might have suggested malignancy. Lastly, in those series in which DM was confirmed but the evaluation, symptoms, or signs were

Site and type of tumor

Breast, adenocarcinoma Breast, adenocarcinoma Uterus, adenocarcinoma Thyroid, carcinoma Breast, scirrhous carcinoma Lung, squarnous cell carcinoma Kidney, Wilms' Breast, adenocarcinoma Cervix, adenocarcinoma Lung, carcinoma Uterus, carcinoma Bladder, transitional cell Bladder, transitional cell Breast, adenocarcinoma a. Uterus, adenocarcinorna b. Colon, adenocarcinoma Colon, adenocarcinoma Breast, adenocarcinoma Breast, adenocarcinoma Colon, adenocarcinoma Breast Breast Breast Ureter, transitional cell Uterine endometrium, adenocarcinoma Uterine endometrium, adenocarcinoma

Duration of tumor to myositis (mo)

24 72 24 72 6 156 84 44 6 4 15 23 2 12 264 24 24 24 156 12 24 3 16 ? 3 24

not available, I wrote the authors for further information regarding their cases; several of the authors replied with the required information, and thus those cases are included. The criteria for the diagnosis of dermatomyositis were the presence of characteristic cutaneous findings with (1) progressive symmetric proximal muscle weakness, (2) a consistent muscle biopsy, (3) a consistent electromyography, and (4) elevated levels of serum muscle enzymes or elevated urinary creatine excretions. In all cases analyzed, malignancy was histologically confirmed. Each case was analyzed regarding the relationship of DM to malignancy, the way in which the malignancy was discovered, and the patient's course. In addition, the symptoms, other than those of DM, were analyzed to find if there were symptoms or signs suggesting the site of malignancy.

Volume 6 Number 2 February, 1982

Malignancy evaluation in dermatomyositis

255

Table II. Dermatomyositis and concurrent malignancy

_

A°, or

I

I

I

I Sox l

I

1. Alexander and Forman lr 2. Montes et al TM

56 51

M M

3. Wilson et a119

54

F

4. Rapoport and Omenn 12 5. Otnes et al2°

76 32

M M

6. Vanderploeg '2~

51

F

s,to a., t,poo, to.o Bladder, transitional cell Metastatic bronchogenic carcinoma a. Metastatic breast adenocarcinoma b. Colon, adenocarcinoma c. Metastatic disease led to recurrence Prostate, metastatic disease Prostate, endometrial carcinoma Metastatic breast adenocarci-

C o m m e n t on discovery

Hematuria Cough, weight loss, chest x-ray Breast mass Rectal bleeding

History of carcinoma Rectal examination Mass

noma

7. TalbotP

71

M

8. Allan et alTM 9. Callen et al'~

41 80

F M

10. Callen et aP

38

F

11. 12. 13. 14. 15. 16.

34 58 46 58 36 60

F F F F M M

Callen et ala Callen et al3 Scaling et al~2 Rose'2a Gray et a124 Present Case 3

Stomach, adenocarcinoma (metastatic) Colon, metastatic to liver Colon, adenocarcinoma (metastatic) Breast, metastatic to tracheal nodes; adenocarcinoma Metastatic, unknown primary Metastatic, unknown primary Ovary, metastatic disease Bronchial, oat cell carcinoma Hepatoma Lung, squamous cell carcinoma

RESULTS

Tables I to III are accumulations of the cases used for analysis in this report. Table I lists those cases in which DM was preceded by the tumor. a-5.9- t6 The tumors occurred from 264 months prior to the onset of DM to 2 months before DM. Nineteen of the patients in whom the sex was known (total of 21) were females. The most common tumors were in the breast (10) and uterus or cervix (6). Three patients had multiple primary tumors occurring with DM. Only three of the patients with preceding tumors had metastatic disease at the onset of their DM. This included one male with metastatic carcinoma of the prostate, a female with metastatic colon adenocarcinoma, and a female with metastatic breast cancer. The remaining twenty-two patients had no evidence of metastatic disease. In all these cases the evidence of malignancy was present by a careful history. In

Unrelenting course of DM; upper GI led to aspiration History Hematochezia History, autopsy, unrelenting course

Pain Pain Pelvic Chest x-ray with tomograms Abnormal liver function tests Chest x-ray

each case, when a second malignancy occurred, it was preceded by suggestive symptoms. Table II lists those patients in whom malignancy was discovered at the same time as the diagnosis of derrnatomyositis (concurrent occurrence), a'4'17-24 In addition, there is a comment on how the malignancy was discovered. Three of the patients listed in this table are also listed in Table I and represent metastatic disease. In only three of the sixteen patients would the traditional screening tests (upper GI, barium enema, intravenous pyelogram) have led to the discovery o f the malignancy. In Patient 3, the presence of rectal bleeding led to the discovery of colonic adenocarcinoma. ~s In Patient 7, in whom an unrelenting course of the dermatomyositis occurred and the cancer was asymptomatic, however, the upper GI was negative because of aspiration of barium which o c -

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Journal of the American Academy of Dermatology

Callen

T a b l e I I I . D e r m a t o m y o s i t i s and malignancy

Site and type of tumor

Duration of myosifis before tumor (too)

Discovery of tumor

1. Fairlamb and Boesen "s

67

M

Lung, oat celt carcinoma

2. Bluefarb et al7 3, Grace and Dao 2~ 4. Brunner and Lobraico 5. Barren '8 6. Levan et arz9 7. Wishart z° 8. Talbott 4

42 41 47

F F F

Uterus, carcinoma Breast, unknown type Ovary, anaplastic carcinoma

96 2 10

57 46 42 49

M M F M

24 6 96 20

9. Talbott 4 10. Talbott 4

72 37

M F

5 144

Dysphagia Acute abdominal symptoms

11, Talbott 4

73

M

Lung, anaplastic carcinoma Nasopharynx squamous cell Reticulosarcoma of vulva Adenocarcinoma, unknown primary Stomach, adenocarcinoma Retroperitoneal mucinous carcinoma Colon, adenocarcinoma

Enlarged axillary nodes, abnormal chest x-ray Vaginal bleeding Breast mass Menorrhagia; abnormal at pelvic examination Cervical lymphadenopathy Nosebleeds Mass Hepatomegaly

138

12. TalbotP

63

F

13. TalbotP

28

M

14, Talbott 4

57

M

Broad ligament, adenocarcinoma Hodgkin's disease, stage IVb Myeloma

Rectal bleeding, abdominal bleeding Postmortem examination

I5. Talbott 4

55

M

Lung, anaplastic

16. Talbott 4 17. TalbotP

45 51

F M

18. Segura and Ziegler 3' 19. Ko et al TM

57 63

F F

20. Callen et al3

61

F

21. Estes et al*

48

F

22. Holmes et a133

44

F

Thyroid, carcinoma Pancreas, metastatic adenocarcinoma Lung, bronchial carcinoma Colon, adenocarcinoma Stomach, adenocarcinoma Metastatic to bone, unknown primary Nasopharynx, squamous cell carcinoma Lung, carcinoma in situ

2 60

Lymphadenopathy

24

101 15

Abnormal protein electrophoresis Cough, fever, abnormal chest x-ray Pain, enlarged thyroid Lymphadenopathy

144 12 15 12

Abnormal chest x-ray Rectal bleeding Rectal bleeding Pain

8

10 3

Dizziness Dyspnea, chest x-ray, bronchoscopy

*Estes S: Nasopharyngealcarcinoma in a patient with dermatomy0sitis. Personal communication,Oct. 1, 1980.

curred. 4 T h e c a n c e r was d i s c o v e r e d at autopsy. In Patient 9, the colonic c a n c e r was suggested by the presence o f hematochezia, a In the remaining patients, the cancer w o u l d not be d i s c o v e r e d by routine screening tests but rather by s y m p t o m s (hematuria, cough, h e m a t o c h e z i a , or pain), by physical findings (breast mass, a b n o r m a l rectal examinations or abnormal pelvic examination), or by an abnormal chest x-ray. A progressive unre-

lenting course of the dermatomyositis despite therapy was present in two patients, and in b o t h metastatic disease was present at autopsy. Table III lists those cases of tumors w h i c h occurred following the diagnosis o f D M . ~'4"2~-a3. T w o of these (3 additional cancers) a r e listed in *Estes S: Nasopharyngeal carcinoma in a patient with dermatomyositls. Personal communication,Oct. 1, 1980.

Volume 6 Number 2 February, 1982 Table I; in each case the cancer following the DM was a second primary, and these three malignancies occurred i, 11,4, and 12 years following the onset of DM. The tumors occurred from 2 months to 144 months following the DM. Twelve of the twenty-two patients were female. In only three would the routine screening tests have led to the discovery of the cancers (stomach, 2; colon, 2), and in these three patients the tumors were discovered by dysphagia (Case 9), continued symptoms of abdominal pain, rectal bleeding (Case 11), and rectal bleeding and anemia (Case 19, 2 primaries). In the remaining eighteen cases the cancers were evident by symptoms (12 malignancies), physical findings (7 malignancies), and abnormal laboratory findings (2 malignancies, 1 with abnormal chest x-ray and 1 with abnormal protein electrophoresis). A progressive or recurrent course of the disease was found with occurrence of the tumor ineleven cases (Cases 2, 4, 8, 9, 10, 12, 16, 17, 20, 21, 22), thus suggesting that this finding may be important in the discovery of tumor; however, in none of the eleven patients was the reappearance or progressive nature of the DM the only symptom which occurred. Table IV lists the tumors which were reported by Barnes and in subsequent reports, t-:~ The sites commonly found in these patients are generally not amenable to screening tests, or can easily be found by physical examination or chest x-ray examination. Of the screening tests available, it would seem that the intravenous pyelogram would be of little value since only five of the 333 cases reported had renal carcinoma. In addition, colon and stomach carcinoma represent less than one sixth of the cases reported, and in the patients examined here the discovery was evident by suggestive symptoms or signs. DISCUSSION

DM has been linked with an increased propensity toward internal malignancy.t'a'4'(l However, this relationship is not uniform: 1. Not all cases of DM are related to malignancy. 2. The malignancy can precede, follow, or occur with DM. 3. There is no unique cell type or site of malignancy.

Malignancy evaluation in dermatomyositis 257

Table IV. Observed tumors with dermatomyositis

Site

I Reported I Subsequent[ by Barnes reports [ Total

Breast Lung Ovary Stomach Colon Uterine Lymphoma--leukemia Nasopharynx Prostate Unknown primary Thyroid Bladder Pancreas Other

46 42 22 21 17 17 " 18 15 10 4 3 2 2 39

11 10 6 5 8 7 2 1 3 5 2 3 2 6

57 52 28 26 25 24 20 16 13 9 5 5 4 45

Total

258

71

329

4. Despite tumor therapy the DM may follow its own course--or DM may be benefitted or worsened by successful tumor therapy. 5. Recurrence of tumor does not always mean a return of the DM, but this can occur. For all these reasons this relationship is:still controversial. Callen et aP showed that thereis an increased incidence compared to polymyositis (PM) and suggested that extensive evaluation was rarely useful in the discovery of malignancy. The value of an extensive radiologic or surgical approach to the patient with DM is controversial. Boharl and Peter" were the first to question this " d o g m a . " Later, Moss and Hanelin 5 and Callen et al, a in their studies, were unable to confirm the value of malignancy evaluation. However, opinion still remains mixed, s'l" Practically speaking, I have reviewed cases in which malignancy search was conducted or ordered on patients in whom a history and physical examination were not completed. Unquestionably, this is poor medical care and cannot be condoned. Thus, in this report I have attempted to analyze the data on which the recommendation for extensive evaluation is based . . . . . . In this report i have reviewed fifty-three cases from the literature in which data were available confirming the diagnosis of malignancy and in

258

Callen

which the case report contained information regarding the discovery of malignancy. In addition, I have included three newly reported personal cases and one case of Dr. Stephen Estes seen by me at a regional meeting. Many cases have not been included; in most cases this was because information regarding the discovery of the malignancy was not available from the report or from a letter to the author. 34 An example is the report of Bohan et al 3'~ in a patient in whom carcinoma of the stomach appeared 3 months following the diagnosis of dermatomyositis; however, it could not be determined how the malignancy was discovered, whether there were abdominal symptoms, hematochezia, iron deficiency anemia, or other findings that would suggest evaluation. Therefore, this case could not be included in this review. Similarly, some reports lack data regarding the time relationship of the malignancy and the dermatomyositis, and thus the six cases of Sheard and Knoefler3~ could not be analyzed. Several naturally occurring factors weigh against the value of malignancy work-up. First, many patients have tumors prior to the diagnosis of dermatomyositis (Table I). Second, some patients have malignancy which is long removed from the presentation of dermatomyositis, at a time when the muscle disease may be quiescent (Table III). Finally, the tumors that do occur are often in areas which can be discovered by physical findings or "routine" laboratory evaluation, such as a chest x-ray examination, blood count, or stool guaiac. Table IV demonstrates that in most of the 333 reported cases, tumors occurred in areas amenable to physical examination (breast, uterus or cervix, prostate, lymphoma, nasopharynx)or "routine" laboratory such as complete blood cell count (leukemia, conditions causing anemia), chest x-ray examination (lung), urinalysis (bladder, kidney), or stool guaiac (colon, stomach). It can be argued that there may be occult neoplasm of the gastrointestinal tract or urinary tract which can be discovered by a "blind" radiologic search. However, if the initial work-up fails to discover a malignancy, when should the clinician reevaluate the laboratory tests? Repeated search in the absence of symptoms did not lead to discovery of malignancy either. One alarming conclusion from this review and

Journal of the American Academy of Derrnatotogy

evident in our previous report is the substitution o f diagnostic tests for the basics of physical examination. In our review of twenty-eight cases of dermatomyositis, pelvic or rectal examinations were not carried out in several patients who had extensive radiologic search, including upper GI, barium enema, intravenous pyelogram, and liver-spleen scans .3 Similarly, Scaling et al 2z reported twenty-five cases in female patients with DM in whom five cases of malignancy were found; of these twentyfive patients, ten had not had a pelvic examination during their admission. Also, one of their patients without malignancy had a vaginal hysterectomy in search of occult malignancy. The use of extensive laboratory tests cannot be justified in the patient in whom physical examination is not complete. The conclusion from the literature reviewed cannot be viewed in the absence of anecdotal information which suggests that "cancer screen" is valuable. In my correspondence with Dr. Vesterager,* he believed that a cancer screen led to the diagnosis of cancer of the stomach and rectal carcinoma in two of their patients. Although they did state that these patients were asymptomatic, they did not state whether the patients had hematochezia, anemia, or progressive nonresponsire myositis. Thus I was unable to completely analyze these cases. They did state in their article that the effects of therapy were similar in their groups of DM with or without malignancy. Lastly, several authors, including myself, have suggested that in the presence of malignancy the DM will not respond to appropriate therapy, or if malignancy recurs the dermatomyositis will flare. 3'6 Unfortunately, these statements are n o t uniformly tree. In this review progressive myositis was evident in only eight cases; metastatic disease led to recurrence in several cases, a'4.r''°,'2''~'2° However, several cases, including Case 1, showed little change in the myositis despite tumor removal. 19,27 This review has analyzed several recent cases along with previously published data. Retrospective studies are often criticized because their data cannot be statistically analyzed in a meaningful fashion. In this study, however, the goal has not *Vesterager L: Personal communication,Aug. 22, 1980.

Volume 6 Number 2 February, 1982

been to prove that DM and cancer are associated, nor to attempt to prove that malignancy work-up is necessary. Conclusions have been drawn from the previous literature which I believe were not rational, and thus I have reviewed the studies from which the conclusions regarding a malignancy search are based. The literature would be benefitted by reports of cases in which malignancy screens were valuable; until such reports appear, I must conclude that the use of extensive radiologic screening tests in patients with dermatomyositis is without value. Directed tests should be conducted in patients with (1) previous malignancy or (2) unexplained laboratory test, unexplained symptoms or signs; possibly they should be conducted in patients with (1) unrelenting course or (2) nonresponders to appropriate therapy. REFERENCES

l. Barnes BE: Dermatomyositis and malignancy. Ann Intern Med 84:68-76, 1976. 2. Bohan A, Peter JB: Polymyositis and dermatomyositis. (Parts I and II.) N Engl J Med 292:344-348; 403-407, 1975. 3. Callen JP, Hyla JF, Bole GG Jr, Kay DR: The relationship of dermatomyositis and polymyositis te internal malignancy. Arch Dermatol 116-295-298, 1980. 4. Talbott JH: Acute dermatomyositis-polymyositis and malignancy. Semin Arthritis Rheum 6:305-360, 1977. 5. Moss AA, l-tanelin LG: Occult malignant tumors in dermatologic disease. Radiology 123:69-71, 1977. 6. Rowland LP, Clark C, Olarte M: Therapy for dermatomyositis and polymyositis. Adv Neurol 17:63-97, 1977. 7. Bluefarb SM, Walk S, Gigli L.: Dermatomyositis. Arch Dermatol 83:168-169, 1961. 8. Tuffanelli DL: Connective tissue diseases, in Malkinson F, Pearson R, editors: Year book of dermatology. Chicago, 1978, Year Book Medical Publishers, Inc., pp. 9-36. 9. Harris PL, Bum I, Scott JT: Dermatomyositis associated with carcinoma of the breast. Proc R Soc Med 68:26, 1975. 10. Kellogg DR, Talley RW: Concurrent dermatomyositis and metastatic breast carcinoma. Henry Ford Hosp Med Bull 14:405-409, 1966. 11. Calvert HT, Neale EJ: Dermatomyositis following treatment of cancer. Postgrad Med J 41:371-373, 1965. 12. Rapoport AH, Omenn GS: Dermatomyositis and malignant effusions: Rare manifestations of carcinoma of the prostate. J Urol 100:183-187, 1968. 13. Vesterager L, Worm AM, Thomsen K: Dermatomyositis and malignancy. Clin Exp Dermatol 5:31-35, 1980. 14. Lintum JCA, Goedbloed R: Poikilodermatomyositis. Br J Dermatol 91:52-53, 1974. 15. Ko HS, Ogryzlo MA, Pruzanski W: Polymyositis in a patient with multiple neoplasms. J Rheumatol 3:233240. 1976.

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16. Allan RN, Dykes PW, Harris OD, Oates GD: Dermatomyositis associated with hepatic secondaries from carcinoma of the colon, Gastroenterology 62:1227-1231, 1972. 17. Alexander S, Forman L: Dermatomyositis and carcinoma: A case report and immunological investigation. Br J Dermatol 80:86-89, 1978. 18. Montes CF, Lascano EF, Alva O, et al: Dermatomyositis and bronchogenic carcinoma. Arch Dermatol 87:637640, 1963. 19. Wilson E, Finley AD, Killingback M: Triple primary carcinoma of rectum, breast, and transverse colon associated with dermatomyositis. Br Med J 2:80-83, 1965. 20. Otnes B, Refsum SB, Husby G, Mathisen W; Probable endometrial carcinoma of the prostate, crossed renal ectopia and dermatomyositis in a 32 year old man. J UrN 120:504-507, 1978. 21. Vanderploeg DE: Dermatomyositis and malignancy. Cutis 19:205-207, 1977. 22. Scaling ST, Kaufman RH, Patten BM: Dermatomyositis and female malignancy. Obstet Gynecol 54:474-476, 1979. 23. Rose JDG: Membranous glomerulonephritis, dermatomyositis and bronchial carcinoma. Br Med J 2:641, 1979. 24. Gray RG, Altman RD, Gotflieb NL: Aberrant serum enzyme patterns in dermatomyositis associated with hepatoma. J Rheumatol 3:227-232, 1976. 25. Fairlamb D, Boesen E: Gyneeomastia associated with gonadotrophin-secreting carcinoma of the lung. Postgrad Meal J 53:269-271, 1977. 26. Grace JT, Dao TL: Dermatomyositis in cancer: A possible etiological mechanism. Cancer 12:648-650, 1959. 27. Brunner MJ, Lobraico RV Jr: Dermatomyositis as an index of malignant neoplasm: A report of a case and review of the literature. Ann Intern Med 34:1269-1273, 1951. 28. Barten RP: Collagen disease and cancer. Radiology 92: 972-974, 1969. 29. Levan NE, Kaplan M, Treiger M: Dermatomyositis with squamous cell carcinoma of the nasopharynx. Arch Dermatol 106:410, 1972. 30. Wishart JM: Retieulosarcoma of the vulva complicating dermatomyositis treated by immunosuppression. Proc R Soc Med 66:330, 1973. 31. Segura R, Ziegler DK: Dermatomyositis. A recurrence of symptoms following treatment of neoplasm. Dis Nerv Syst 34:284-286, 1973. 32. Schwarz HA, Slavin G, Ward P, Ansell BM: Muscle biopsy in polyrnyositis and dermatomyositis: A clinicopathological study. Ann Rheum Dis 39:500-507, 1980. 33. Holmes R, Black MM, Farebrother MJB, Van Grutten M: Malignancy associated dermatomyositis with fibrosing alveolitis. Clin Exp Dermatol 5:415-420, 1980. 34. DeVere R, Bradley WG: Polymyositis: its presentation, morbidity and mortality. Brain 98:637-666, 1975. 35. Bohan A, Peter JB, Bowmar RL, Pearson CM; A computer-assisted analysis of 153 patients with polymyositis and dermatomyositis. Medicine 56:255-286, 1977. 36. Sheard C, Knoefler PT: Dermatomyositis and the incidence of associated malignancy. Arch Dermatol 75: 224-227, 1957.